Fumarate Hydratase / FH抗体

Fumarate Hydratase / FH抗体产品信息

免疫原 :
Recombinant Human Fumarate Hydratase / FH protein (Catalog#12115-H08E)
Antibody Type : Mouse Monoclonal Antibody ( Mouse mAb Service Platform )
克隆号 :
3D5A3G7
抗体宿主 :
Mouse IgG1
缓冲液 : 0.2 μm filtered solution in PBS, 5% trehalose may be added in some batches. Please read the hardcopy of COA or contact our customer service to confirm the formulation.
制备方法 :
This antibody was produced from a hybridoma resulting from the fusion of a mouse myeloma with B cells obtained from a mouse immunized with purified, recombinant Human Fumarate Hydratase / FH (rh Fumarate Hydratase / FH; Catalog#12115-H08E; P07954-1; Ala 45-Lys 510). The IgG fraction of the cell culture supernatant was purified by Protein A affinity chromatography.
Fumarate Hydratase / FH抗体Background
Fumarate hydratase, also known as Fumarase and FH, is a protein which belongs to theclass-II fumarase / aspartase family and fumarase subfamily. The phenomenon of enhanced glycolysis in tumours has been acknowledged for decades, but biochemical evidence to explain it is only just beginning to emerge. Succinate dehydrogenase (SDH) and fumarate hydratase (FH) are tumour suppressors and which associated, mitochondrial enzymes and their dysfunction with tumorigenesis. Fumarate hydratase / FH immunohistochemistry may also serve as a useful tool to screen for hereditary leiomyomatosis and renal cell cancer (HLRCC) in young individuals with clear cell renal carcinoma. Defects in FH are the cause of fumarase deficiency (FHD) which also known as fumaricaciduria. FHD is characterized by progressive encephalopathy, developmental delay, hypotonia, cerebral atrophy and lactic and pyruvic acidemia. Defects in FH are also the cause of multiple cutaneous and uterine leiomyomata (MCUL1) which is an autosomal dominant condition in which affected individuals develop benign smooth muscle tumors (leiomyomata) of the skin. Affected females also usually develop leiomyomata of the uterus (fibroids).
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